NR AIQW
AU Muramatsu,Y.; Shinagawa,M.
TI [A trend of molecular genetics on prion diseases and prion protein]
QU Nippon Rinsho. Japanese Journal of Clinical Medicine 1993 Sep; 51(9): 2494-502
PT journal article; review; review literature
AB Infectious amyloid filaments designated as prion rods or scrapie associated fibrils (SAF) present in brain tissues affected by transmissible spongiform encephalopathies such as Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker disease (GSS) and kuru of humans, and scrapie of sheep. A hydrophobic glycoprotein, PrPsc is a major component of SAF, and is known to be associated with the infectivity of these diseases. Both PrPsc and the normal isoform of this glycoprotein, PrPc are encoded by a single host gene, PrP gene, and the conversion of PrPc to PrPsc is a posttranslational event. Several mutations on the PrP gene are associated with variations of the phenotype and the occurrence in familial CJD and GSS.
ZR 44
MH Animal; Codon; Creutzfeldt-Jakob Syndrome/genetics/pathology; English Abstract; Gerstmann-Sträussler-Scheinker Disease/genetics/pathology; Human; Membrane Glycoproteins/genetics; Mutation; Prion Diseases/*genetics; Prions/*genetics; Sheep
AD Department of Veterinary Public Health, School of Veterinary Medicine, Obihiro University of Agriculture and Veterinary Medicine.
SP japanisch
PO Japan